INFORMATION AVAILABLE IN ENGLISH, GUJARATI AND HINDI
Exomphalos is a type of abdominal wall defect. It occurs when a child’s abdomen does not develop fully while in the womb.
Early in all pregnancies, the intestine develops inside the umbilical cord and then usually moves inside the abdomen a few weeks later. In exomphalos, the intestines and sometimes other organs such as the liver, remain inside the umbilical cord but outside the abdomen.
What causes exomphalos?
We do not know what causes exomphalos. It affects two in every 5,000 children born each year. Exomphalos can be associated with other problems, but the doctors will examine your child closely to check if this is the case.
What are the signs and symptoms of exomphalos?
Exomphalos is immediately recognizable because the child’s intestines are outside the body and covered in a membrane. The size of the bulging membrane containing the intestines and other organs varies from a small protrusion to quite a large lump.
There are two types of exompahlos:
Exomphalos minor where the opening is less than 4cm and only containing the intestine, and Exomphalos major where the opening is greater than 4cm and/or with the liver inside the cord.
How is exomphalos diagnosed?
In many cases, exomphalos is visible on pre-natal ultrasound scanning, which is useful because it gives time for discussions and planning for when and where to give birth.
How is exomphalos treated?
Exomphalos is a serious condition so needs prompt treatment soon after birth.
Depending on the size of the exomphalos, your child may need to have it repaired in one operation or in several stages. If the exomphalos is small and the child is stable, they may have an operation soon after admission to the hospital, where the surgeon replaces the contents back inside the abdomen and closes up the base of the umbilical cord.
If the exomphalos is larger, contains the liver and/or your child needs to be stabilised, doctors may place a silo or pouch over the intestines, which is closed over a period of days to weeks, to allow your child to grow so that there is room inside the abdomen.
What does the operation involve?
If your child is having a one-stage repair under general anaesthetic, the surgeons will replace your child’s entire intestine into the abdominal space and close up the hole at the base of the umbilical cord.
Sometimes, they may need to use a ‘patch’ of material if the hole is quite large. They will cover the area with a dressing to protect the wound while it heals.
In a staged repair, there is too much intestine outside of the abdomen to put back without causing further damage or the space inside the abdomen is too tight. While your child is under general anaesthetic, the surgeon will make a mesh sac and put it over the intestine which keeps it contained and protected.
This sac is then suspended above your child so that gravity gradually moves the intestines back inside the abdomen. It is tightened regularly until all the intestine is inside the abdomen, which usually takes a few days to a few weeks. Your child will then have an operation under general anaesthetic to close up the skin and muscles.
Are there any risks with the operation?
All surgery carries a small risk of bleeding during or after the operation. During the operation, the surgeon will minimise any bleeding by sealing off the blood vessels affected. There is a very small chance that nearby structures in the abdomen could be damaged during surgery but this is a very rare occurrence.
Every anaesthetic carries a risk of complications, but this is very small. Your child’s anaesthetist is a very experienced doctor who is trained to deal with any complications.
It can take a while after the operation for the intestine to start working properly so your child may need to be fed intravenously using total parenteral nutrition (TPN) for a while. Some babies with exomphalos have breathing problems which may require more support for a longer period.
Are there any alternatives to the operation?
In some scenarios, the child may be treated with particular chemicals / ointments in order to make the membrane covering the intestine hard. The surgery may hence be delayed by a few weeks. Ultimately, the condition needs to be treated to allow your child to grow and develop.
What happens after the operation?
Your baby will come back to recover either in the intensive care unit .All babies are closely monitored after the operation, and so your baby will be connected to monitors to check their breathing, heart rate and oxygen levels. If your child needs help with breathing, they will be connected to a ventilator. They will also be given pain relief through the intravenous infusion (drip).
What happens next?
The outlook for children born with exomphalos varies depending on the size of the defect and any other problems. Many children have grown up to live normal lives.
Children who have had aexomphalos repair may develop hernias in the years after the operation. This is because the abdomen has fewer muscles than usual.
GUJARATI
એક્ઝોમ્ફેલોસ
એક્ઝોમ્ફેલોસ ૫૦૦૦ એ ફક્ત ૨ બાળકોમાં જાવા મળતી એક ગંભીર જન્મજાત ખોડ છે.
સામાન્ય રીતે ગર્ભમાંના બાળકનાં આંતરડા તેના પેટની બહાર આકાર લઈને નાળના માર્ગેથી પેટની અંદર જતાં હોય છે. આ ખોડમાં બાળકના પેટની દીવાલ બરાબર ન બનતા તેના આંતરડા અને કોઈક વખતે બાળકનું લીવર પેટની બહાર નાળની અંદર ફસાયેલા રહેતા હોય છે.
આ ખોડ બાળકને જાતાં જ ખબર પડી જાય છે. બાળકના આંતરડા પેટની બહાર પારદર્શક પડથી ઢંકાયેલા દેખાય છે. બાળકને તાત્કાલિક સારવારની જરૂર પડે છે. સારવાર અર્થે મોકલતા પહેલાં બાળકનાં આંતરડાને ચોખ્ખા સલાઈનવાળા કાપડથી ઢાંકી દેવું જાઈએ. આ બાળકોમાં ઓપરેશનની જરૂર પડતી હોય છે. આૅપરેશન એક સ્ટેજ અથવા બે સ્ટેજમાં કરવામાં આવે છે. જા બાળક આૅપરેશન ખમી શકવાની Âસ્થતિમાં ન હોય તો અમુક કેમિકલ/દવાઓ લગાવી આંતરડાના ઉપરના પડને કડક કરવામાં આવે છે જેથી બાળકને ઇન્ફેક્શન ન લાગે. આ બાદ યોગ્ય સમયે આૅપરેશન કરી પેટની દીવાલ બનાવવામાં આવે છે. •
HINDI
एक्जोमफेलस (नाभि सम्बंधी जन्मनात हर्निया)
एक्ज़ोमफेलस पेट की दीवार का एक दोष है। हर ५००० में २ बच्चों को यह प्रभावित करता है। गर्भाधान के कुछ ही सप्ताह बाद, गर्भनाल के भीतर आंतों का विकास होता है। एक्ज़ोमफेलस में आंतें और कभी-कभी अन्य अंग जैसाकि जीगर गर्भनाल के भीतर परंतु उदर के बाहर रह जाते हैं। एक्ज़ोमफेलस के बारे में तुरंत पता लग जाता है क्योंकि बच्चे की आंते शरीर के बाहर होती है और इनपर एक आवरण होता है। जन्म से पहले ही अल्ट्रासाउंड के द्वारा एक्ज़ोमफेलस का पता चल जाता है। एक्ज़ोमफेलस एक गंभीर परिस्थिति है और जन्म के तुरंत बाद इसका उपचार किया जाना आवश्यक होता है।
प्रसूति करानेवाले फिजीशीयन को सावधानीपूर्वक इन्हें (आंतो को) पानी में भीगोए जंतुरहित कपड़े से ढंक देना चाहिए और बच्चे को बालविशेषज्ञ के पास स्थानांतरित करना चाहिए।
एक्ज़ोमफेलस के आकार के आधार पर, एक ऑपरेशन अथवा अनेक स्तरीय ऑपरेशन के द्वारा इसे ठीक करने की आवश्यकता हो सकती है। यदि एक्ज़ोमफेलस अधिक बड़ा है, उसमें जीगर शामिल है और/अथवा बच्चे को स्थायीकरण की आवश्यकता हो, तो सर्जन को आंतों के ऊपर कुशूल अथवा थैली रख देनी चाहिए, ताकि बच्चा बढ़ सके और उदर के भीतर अधिक जगह बन सके। कुछ मामलों में, उपचार विशेष रसायनों/मलमों के द्वारा किया जाना चाहिए जिससे कि आंतों के ऊपर का आवरण सख्त हो जाए। तदनुसार, सर्जरी को कुछ हफ्तों के लिए विलम्बित किया जाना चाहिए। एक्ज़ोमफेलस के साथ जन्मे बच्चे का दृष्टिकोण विसंगति के आकर और अन्य समस्याओं के अनुसार भिन्न हो सकता है। कई बच्चे बड़े होकर सामान्य जीवन व्यतीत करते हैं। •
